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BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome characterized by the triad of cutaneous hemangiomas, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. Clinical manifestations, genetic testing, and radiologic imaging are the key steps in diagnosing this syndrome. CASE DESCRIPTION: An 18-month-old boy was brought for follow-up brain magnetic resonance imaging (MRI) with a history of right lower limb hypertrophy, cutaneous varicosities, and hemangiomas diagnosed at birth. A baseline MRI at 12 months revealed multiple hemorrhagic lesions within the cerebrum, the largest in the right temporal lobe, which was treated surgically at the age of 18 months because of its rapid growth. This is the youngest patient with KTWS treated surgically for intracranial hemangiomas. CONCLUSION: KTWS is a rare disease with a wide range of manifestations. Multisystemic evaluation of this group of patients should be performed to identify cavernous hemangiomas at the early stage of life and adequately treat them in the future. Treatment of KTWS patients with cavernous hemangiomas should not be different from the treatment of patients with any other hemangiomas, and surgical intervention should be considered on a case-to-case bases.

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OBJECTIVE: Our purpose was to investigate the added diagnostic value of C-arm contrast-enhanced cone-beam CT (CE-CBCT) to routine contrast-enhanced MRI (CE-MRI) in detecting associated developmental venous anomalies (DVAs) in patients with sporadic intracranial cavernous malformations (ICMs). METHODS: Fifty-six patients (53 with single and three with double ICMs) met the inclusion criteria. All patients had routine CE-MRI scans performed at 1.5 Tesla. The imaging studies (CE-MRI and CE-CBCT) were retrospectively and independently reviewed by two observers, with consensus by a third. Group difference, intra- and interobserver agreement, and diagnostic performance of the modalities in detecting associated DVAs were calculated. Reference standard was CE-MRI. RESULTS: On CE-MRI and CE-CBCT, 37 (66%; of 56) and 47 patients (84%; of 56) had associated DVAs, respectively. In 10 patients (52.6%; of CE-MRI negatives [n=19]), CE-CBCT improved the diagnosis. Nine patients (16%; of 56) had no DVA on both imaging techniques. Difference in proportions of associated DVAs on CE-MRI and CE-CBCT was statistically significant, p < 0.05. Sensitivity, specificity, positive likelihood ratio, and area under the curve of CE-CBCT were 100% (95% confidence interval [CI]: 90.5-100%), 47.3% (95% CI: 24.4-71.1%), 1.9 (95%CI: 1.240-2.911), 0.737 (95%CI: 0.602-0.845), respectively. Intraobserver agreement was excellent for CE-MRI, kappa (κ) coefficient = 0.960, and CE-CBCT, κ=0.931. Interobserver agreement was substantial for CE-MRI, κ=0.803, and excellent for CE-CBCT, κ=0.810. CONCLUSIONS: CE-CBCT is a useful imaging technique especially in patients with negative routine CE-MRI in terms of detecting associated DVAs. In nearly half of these particular patients, it reveals an associated DVA as a new diagnosis. KEY POINTS: • Although it is known to be the gold standard, some of the DVAs associated with ICMs are underdiagnosed with CE-MRI. • In nearly half of the patients with negative routine CE-MRI, CE-CBCT reveals an associated DVA as a new diagnosis. • Intra- and interobserver agreement on CE-CBCT is excellent in terms of detecting associated DVAs.

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La anomalía venosa del desarrollo intracraneal (AVD) representa la malformación vascular intracraneal más frecuente. En la inmensa mayoría de los casos es incidental y asintomática, y se considera benigna. No obstante, muy excepcionalmente puede presentarse con clínica neurológica. En este trabajo se presentan tres casos de pacientes con AVD que iniciaron distinta sintomatología debida a complicaciones derivadas de alteraciones en el drenaje venoso. Dichas AVD se localizaron en la ínsula izquierda, el lóbulo temporal derecho y el cerebelo. La excepcionalidad de los casos presentados, así como de las imágenes asociadas que objetivan el mecanismo productor de la clínica, radica en la baja incidencia de AVD sintomáticas descritas en la literatura

Intracranial developmental venous anomalies are the most common vascular malformation. In the immense majority of cases, these anomalies are asymptomatic and discovered incidentally, and they are considered benign. Very exceptionally, however, they can cause neurological symptoms. In this article, we present three cases of patients with developmental venous anomalies that presented with different symptoms owing to complications derived from altered venous drainage. These anomalies were located in the left insula, right temporal lobe, and cerebellum. The exceptionality of the cases presented as well as of the images associated, which show the mechanism through which the symptoms developed, lies in the low incidence of symptomatic developmental venous anomalies reported in the literature

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BACKGROUND: Developmental venous anomaly (DVA) or venous angioma is a common anomaly of cerebral veins that is found incidentally in the majority of cases. There are few cases of arteriovenous shunting in DVA associated with a more malignant course of the disease. Whether these DVAs with shunts are of congenital pathology or lifetime formations is unclear. CASE DESCRIPTION: We report a case of lifetime arteriovenous shunt formation in DVA that caused intracerebral hemorrhage in a child. The patient underwent 2 sequential direct surgeries: an emergency evacuation of the intracerebral hematoma and a scheduled excision of the DVA with arteriovenous shunting. CONCLUSIONS: Arteriovenous shunting in DVA may develop during a lifetime and cause intracerebral hemorrhages. This case showed that localization of DVA with arteriovenous shunting in a noneloquent area enables its complete microsurgical excision with favorable functional outcomes.

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AIM: To determine clinical manifestations, imaging findings and outcome of patients with thrombosed developmental venous anomalies (DVAs). MATERIALS AND METHODS: The radiology database was searched retrospectively for thrombosed DVAs between 01/01/2000 to 07/01/2016. Demographic variables, associated risk factors, clinical manifestations, imaging findings, treatments, and follow-up were recorded. RESULTS: Six patients were found (four female and two male; age range 16-45 years with mean age, 21.3 years). The most common clinical presentation was headaches followed by neurological deficits and seizures. Venous infarction, parenchymal haemorrhage, venous congestive oedema were noted as the radiological findings. Clinical outcome was favourable in all patients with complete recovery or persistence of mild neurological symptoms. CONCLUSION: Thrombosed DVAs may occur under rare circumstances, which lead to variable symptoms. Familiarity with this entity and early recognition of associated findings including venous infarction, parenchymal haemorrhage, and venous congestive oedema would help early diagnosis and prompt treatment.

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Various mixed associations between arteriovenous malformations, cavernous malformations, developmental venous anomalies, and capillary telangiectasias have been described, and a common pathophysiologic event has been suggested to be present, although it is yet to be elucidated. We depict herein the imaging features of a patient who presented with a spontaneous cerebellar hemorrhage, in whom radiologic studies demonstrated a pontine telangiectasia, a brainstem/cerebellar developmental venous anomaly, and a cerebellar proliferative angiopathy. This unique, not previously reported combination of lesions shows that the spectrum of mixed vascular malformations continues to expand. A pathophysiologic mechanism related to the angiogenesis seen in these malformations is also hypothesized.

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Intracranial developmental venous anomalies are the most common vascular malformation. In the immense majority of cases, these anomalies are asymptomatic and discovered incidentally, and they are considered benign. Very exceptionally, however, they can cause neurological symptoms. In this article, we present three cases of patients with developmental venous anomalies that presented with different symptoms owing to complications derived from altered venous drainage. These anomalies were located in the left insula, right temporal lobe, and cerebellum. The exceptionality of the cases presented as well as of the images associated, which show the mechanism through which the symptoms developed, lies in the low incidence of symptomatic developmental venous anomalies reported in the literature.

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Developmental venous anomalies (DVAs) are usually asymptomatic. We report a case of DVA thrombosis with recurrent tiny frontal hematoma in a 24-year-old man. The contribution of T2-GRE and SWAN sequences are discussed. Follow-up attested complete recanalization after anticoagulation.

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Developmental venous anomalies (DVAs) are relatively common lesions, present in up to 3% of the population. The defining characteristic of these lesions is the confluence of radially oriented veins into a single dilated venous channel. DVAs are also known as cerebral venous angiomas, cerebral venous malformations, and cerebral venous medullary malformations. They are the most common type of cerebral vascular malformation found on autopsy studies, and they are often encountered as incidental findings on neuroimaging studies. DVAs are congenital lesions thought to arise from aberrations that occur during venous development, but continue to provide the normal venous drainage to the cerebral territory in which they reside. Although the natural history of DVAs is benign, they may be associated with cavernous malformations or other vascular abnormalities, which can lead to hemorrhage in the vicinity of the DVA. Surgical or endovascular obliteration of DVAs carries a significant risk of venous infarction; thus, conservative management is the treatment of choice for patients with these lesions.

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Developmental venous anomalies (DVAs), previously called venous angiomas, are the most frequently encountered cerebral vascular malformations. However, DVA is considered to be rather an extreme developmental anatomical variation of medullary veins than true malformation. DVAs are composed of dilated medullary veins converging centripetally into a large collecting venous system that drains into the superficial or deep venous system. Their etiology and mechanism are generally accepted that DVAs result from the focal arrest of the normal parenchymal vein development or occlusion of the medullary veins as a compensatory venous system. DVAs per se are benign and asymptomatic except for under certain unusual conditions. The pathomechanisms of symptomatic DVAs are divided into mechanical, flow-related causes, and idiopathic. However, in cases of DVAs associated with hemorrhage, cavernous malformations (CMs) are most often the cause rather than DVAs themselves. The coexistence of CM and DVA is common. There are some possibilities that DVA affects the formation and clinical course of CM because CM related to DVA is generally located within the drainage territory of DVA and is more aggressive than isolated CM in the literature. Brain parenchymal abnormalities surrounding DVA and cerebral varix have also been reported. These phenomena are considered to be the result of venous hypertension associated with DVAs. With the advance of diagnostic imagings, perfusion study supports this hypothesis demonstrating that some DVAs have venous congestion pattern. Although DVAs should be considered benign and clinically silent, they can have potential venous hypertension and can be vulnerable to hemodynamic changes.

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OBJECTIVE: To determine the incidence of fenestrations from multislice spiral computed tomography angiography (CTA) series and to establish the possible association of fenestrations with aneurysms and other vascular diseases of the brain. MATERIAL AND METHODS: Four hundred and eighty-six persons who had undergone brachiocephalic artery (BA) CTA for different clinical indications were selected for retrospective analysis. Postprocessor data processing was made to detect extra- and intracranial artery fenestrations. Saccular and fusiform cerebral aneurysms, arteriovenous malformations (AVMs), and venous angiomas were detected in addition to fenestrations. When aneurysms were concurrent with fenestrations, their locations were compared. RESULTS: Among the 486 patients, fenestrations were revealed in 50 (10.3%) cases, of which there were 46 (9.5%) intracranial artery fenestrations and 4 (0.8%) vertebral artery (VA) ones in the extracranial segments. Aneurysms of different locations were found in 163 (33.5%) patients. Venous angiomas of different locations were seen in 9 (1.9%) persons; AVMs were observed in 21 (4.3%) patients. Fifty-four multiple fenestrations were identified. The bulk of fenestrations were located in the area of the anterior communicating artery (ACA) in 28 (51.9%) patients. Aneurysms were found in 11 (22%) patients with fenestrations. Aneurysms were detected in 152 (34.9%) of 436 patients without fenestrations. The incidence of aneurysms was not statistically different in patients with and without fenestrations (p > 0.05); moreover, aneurysms were somewhat more common in patients without fenestrated vessels. Aneurysm located in the area of fenestration was in only one (9.1%) patient. In other cases aneurysms were located in the vessels having no fenestrations. Aneurysms were relatively frequently encountered in 5 (45.5%) patients with BA fenestration. There was no statistical difference in the incidence of AVMs in the fenestration and non-fenestration groups. Venous angiomas significantly more frequently occurred in patients with fenestrations (p < 0.05). CONCLUSION: CTA findings showed fenestrations 10.3% of patients. Fenestrations were located most frequently in the area of ACA and less frequently in the area of BA and middle cerebral artery. Those of other locations, concomitant and extracranial ones, were very rare. According to our data, there was no direct relationship of fenestrations to arterial aneurysms and AMVs. When fenestrations were in the area of BA, the incidence of aneurysms increased up to 45.5%. Venous angiomas occurred more commonly in patients with fenestrations.

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Developmental venous anomalies (DVA) drain normal neural tissue and are mostly discovered incidentally. We describe a young patient with a left hemisphere superficial to deep DVA and right hemisphere venous outflow restriction presenting with a seizure. The right hemisphere drainage variation is not typical of a DVA but represents another drainage pattern on the border of normality.

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Venous angiomas are a developmental anomaly in which embryonic venous drainage is still present into adulthood. They are usually asymptomatic and benign course but they can cause seizures and less commonly bleeding, usually associated to cavernous malformation. Normally, treatment is not necessary although bleeding, severe clinical and lesions in which it is possible a favourable approach, we can consider treatment. We show a case of a 11 years old boy with acute decrease level of consciousness. We observed hematoma in the right cerebellar hemisphere with radial tubular structures consistent with developmental venous anomaly. The hematoma was evacuated without a demonstrable other reason justifying the bleeding.

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Intercavernous communication between the left and right internal carotid arteries is an uncommon entity. The authors report a case involving a pediatric patient who was found to have such a vascular anomaly. Such variations should be known by the neurosurgeon so as to avoid misdiagnosis and potential iatrogenic injury.

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INTRODUCTION: Cerebral developmental venous anomalies (DVAs) are the most frequently encountered cerebral vascular malformation. As such, they are often observed incidentally during routine CT and MRI studies. Yet, what DVAs represent from a clinical perspective is frequently not common knowledge and DVAs, therefore, still generate uncertainty and concern amongst physicians. This article reviews our current understanding of developmental venous anomalies. RESULTS: In the majority of cases, DVAs follow a benign clinical course. On rare occasions, DVAs become symptomatic generally due to an underlying associated vascular malformation such as cavernous malformations or thrombosis of the collecting vein. Rare forms of DVAs include arterialized DVAs and DVAs involved in the drainage of sinus pericranii, which warrant additional investigation by digital subtraction angiography. Cerebral abnormalities such as atrophy, white matter lesions and calcifications within the drainage territory of asymptomatic DVAs, are often identified on CT or MR imaging studies and likely represent secondary changes due to venous hypertension. There is increasing evidence that DVAs have a propensity for developing venous hypertension, which is thought to be the cause of associated cavernous malformations and parenchymal abnormalities. CONCLUSIONS: DVAs represent variations of the normal cerebral venous angioarchitecture and by enlargement follow an uneventful clinical course. Complications can, however, occur and their management requires a thorough understanding of the nature of DVAs, including their frequent coexistence with other types of vascular malformation, and the existence of more complex but rare forms of presentation, such as the arterialized DVAs.

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We present the case of a 58-year-old man who suffered a left thalamic intracerebral hemorrhage. Brain magnetic resonance imaging (MRI) revealed an incidental venous angioma in the left frontal lobe. Further elucidated by cerebral angiography and functional MRI, this venous angioma exhibited arteriovenous shunting. The arterialized venous angioma represents an uncommon, "mixed" intracranial vascular lesion whose natural history remains unknown.

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